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Marfan syndrome ectopia lentis lens extraction

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Title: Marfan syndrome ectopia lentis lens extraction
Author (s): Russell Swan, MD; Alan Crandall, MD
Date: 01/01/2016
Keywords/Main Subjects: Cataract, Ectopia lentis; Marfan; Phacoemulsification; CTR; CTS
Diagnosis: Marfan’s syndrome; Ectopia lentis; Cataract

This case is from a 5 year old girl who presented to ophthalmology clinic with decreased vision in both eyes, known history of Marfan’s syndrome who was found to have bilateral subluxated natural lenses. After a long discussion with the family they elected to proceed with lens extraction and intraocular lens placement.

This video demonstrates the difficulty associated with these cases due to the diffuse zonulopathy often found. While a femto-assisted capsulorhexis could have helped in this case, the patient’s age and need for general anesthesia prevented the use of this technology at our institution. Given the age of the patient and the need for general anesthesia the decision was made to perform bilateral same day surgery.

After a capsulorhexis is initiated, capsular tension hooks are used to facilitate completion of a continuous curvilinear capsulorhexis. Gentle hydrodissection is carried out and an I/A hand piece is used to remove the crystalline lens. A capsular tension ring is placed in the capsular bag. A conjunctival peritomy is completed and then two adjacent sclerotomy sites are created with a bent 25-gauge trochar. An Ahmed capsular tension segment is threaded with 8-0 Gore-Tex and placed in the anterior chamber. Each end of the Gore-Tex is externalized through the sclerotomy sites. These capsular tension devices are utilized to secure the capsular bag to the sclera and provide support for safe intraocular lens placement. The IOL is placed in the sulcus. Once the IOL is in an appropriate position, the Gore-Tex is secured.  Given the myopic shift over time in this patient +2.0 diopters of hyperopia was targeted. Sulcus fixation was selected given that this would make potential IOL exchange easier in the future if the patient developed significant myopic shift. A 3-piece lens was thus selected as to reduce IOL related complications from the sulcus placement.

It should be noted that there are many surgical approaches to young patients with ectopia lentis related to Marfan syndrome. These include but are not limited to lensectomy with or without anterior vitrectomy, pars plana vitrectomy and lensectomy. The patient can be left aphakic, or corrected with an iris fixated IOL, anterior chamber IOL, or scleral fixated posterior chamber IOL.

Format: video
Crema, A. et al. Femtosecond Laser-assisted Cataract Surgery in Patents With marfan Syndrome and Subluxated Lens. Journal Refractive Surgery. 2015 May: 31(5): 388-41
Miraldi, U., Coussa, R., Traboulsi, E. Surgical management of lens subluxation in Marfan syndrome. Journal AAPOS. 2014 Apr: 18(2):140-6
Identifier: Moran_CORE_20240
Faculty Approval by: Alan Crandall, MD

Russell Swan, MD, ©2015. For further information regarding the rights to this collection, please visit: